Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy

Neurology. 2016 Mar 1;86(9):800-7. doi: 10.1212/WNL.0000000000002418. Epub 2016 Feb 3.


Objective: We report the clinical and serologic features of Japanese patients with chronic inflammatory demyelinating polyneuropathy (CIDP) displaying anti-neurofascin-155 (NF155) immunoglobulin G4 (IgG4) antibodies.

Methods: In sera from 533 patients with CIDP, anti-NF155 IgG4 antibodies were detected by ELISA. Binding of IgG antibodies to central and peripheral nerves was tested.

Results: Anti-NF155 IgG4 antibodies were identified in 38 patients (7%) with CIDP, but not in disease controls or normal participants. These patients were younger at onset as compared to 100 anti-NF155-negative patients with CIDP. Twenty-eight patients (74%) presented with sensory ataxia, 16 (42%) showed tremor, 5 (13%) presented with cerebellar ataxia associated with nystagmus, 3 (8%) had demyelinating lesions in the CNS, and 20 of 25 (80%) had poor response to IV immunoglobulin. The clinical features of the antibody-positive patients were statistically more frequent as compared to negative patients with CIDP (n = 100). Anti-NF155 IgG antibodies targeted similarly central and peripheral paranodes.

Conclusion: Anti-NF155 IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset, ataxia, tremor, CNS demyelination, and a poor response to IV immunoglobulin. The autoantibodies may serve as a biomarker to improve patients' diagnosis and guide treatments.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Aged
  • Aged, 80 and over
  • Autoantibodies / blood*
  • Autoantibodies / immunology
  • Biomarkers / blood*
  • Cell Adhesion Molecules / blood*
  • Cell Adhesion Molecules / immunology
  • Child
  • Female
  • Humans
  • Immunoglobulin G / blood*
  • Immunoglobulin G / immunology
  • Incidence
  • Japan / epidemiology
  • Male
  • Middle Aged
  • Nerve Growth Factors / blood*
  • Nerve Growth Factors / immunology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / blood*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / epidemiology*
  • Risk Assessment / methods
  • Young Adult


  • Autoantibodies
  • Biomarkers
  • Cell Adhesion Molecules
  • Immunoglobulin G
  • NFASC protein, human
  • Nerve Growth Factors